Abstract
The applications of disease cluster investigations in medicine have developed rather rapidly in recent decades. Analyzing the epidemiology of non-random aggregation of patients with a particular disease fostered identification of environmental and external exposures as disease triggers and promoters. Observation of patient clusters and their association with nearby exposures, such as Dr. John Snow's astute mapping analysis in the mid-1800's, which revealed proximity of cholera patients in London to a contaminated water pump infected with Vibrio cholerae, have paved the way for the field of epidemiology. This approach enabled the identification of triggers for many human diseases including infections and cancers. Cutaneous T-cell lymphomas (CTCL) represent a group of non-Hodgkin lymphomas that primarily affect the skin. The detailed pathogenesis by which CTCL develops remains largely unknown. Notably, non-random clustering of CTCL patients was reported in several areas worldwide and this rare malignancy was also described to affect multiple members of the same family. These observations indicate that external factors are possibly implicated in promoting CTCL lymphomagenesis. Here, we review the epidemiology of CTCL worldwide and the clinical characteristics of CTCL patients, as revealed by global epidemiological data. Further, we review the known risk factors including sex, age, race as well as environmental, infectious, iatrogenic and other exposures, that are implicated in CTCL lymphomagenesis and discuss conceivable mechanisms by which these factors may trigger this malignancy.
Highlights
While mycosis fungoides (MF) and Sézary syndrome (SS) are the most commonly recognized subtypes, other variants of Cutaneous T-cell lymphomas (CTCL) were documented by the 2016 World Health Organization classification of primary cutaneous lymphomas [4] and include angioimmunoblastic T-cell lymphoma, subcutaneous panniculitis-like T-cell lymphoma, adult T-cell leukemia/lymphoma (ATLL), mature T-cell lymphoma not otherwise specified (NOS), cluster of differentiation 30 positive (CD30+) T-cell lymphoproliferative disorders of the skin and extranodal natural killer (NK)/T-cell lymphoma, nasal type (ENKL)
Given the evidence of geographical regional clustering of CTCL patients, CTCL occurrence in unrelated family members and recent evidence implicating S. aureus in the pathogenesis/progression of CTCL, more research is needed to decipher the precise mechanism by which specific environmental exposures may be driving the pathogenesis of this malignancy
Such knowledge of potential triggers and perpetuating factors for this cancer would enable us at some point to significantly decrease CTCL incidence
Summary
Non-random clustering of CTCL patients was reported in several areas worldwide and this rare malignancy was described to affect multiple members of the same family. In one study that examined the clinical characteristics of 4,496 patients diagnosed with cutaneous lymphoma between 2004 and 2008 in the United States, the mean age at the time of diagnosis for Caucasian, African-American Asian/Pacific Islander, and Native American/other/unknown patients were 59.2, 51.5, 51.3, and 53.8 years, respectively [9].
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